Primary adenoid cystic carcinoma in the esophagus.

Esophageal adenoid cystic carcinoma (EACC) is an exceedingly rare malignant tumor constituting only 0.2% of all esophageal tumors. The tumor exhibits aggressive behavior, composed histologically of ductal and modified myoepithelial cells. We report a case of a 69-year-old female with a diagnosis of an EACC by preoperative endoscopic biopsy. Thoracoscopy esophagectomy was carried out. However, pleural metastasis was found. Therefore, surgical resection of the esophageal tumor was not carried out. The patient underwent an uneventful recovery, followed by palliative treatment and ongoing chemoradiotherapy. EACC is uncommon but exhibits a more aggressive nature compared to its counterparts in the head and neck region. Dysphagia associated with gastroesophageal reflux disease is a common symptom. The duration from symptom onset to diagnosis is typically short. Treatment options include surgical resection, chemotherapy, and radiotherapy, with surgery being the preferred initial approach despite high operative mortality. Prognosis remains inconclusive, with some studies associating poor outcomes with lymph node metastasis and vascular invasion, while others report better survival rates. EACC presents diagnostic and therapeutic challenges due to its rarity and aggressive nature. Prognostic considerations remain unclear, emphasizing the need for further research and accumulated cases to delineate optimal treatment. The presented case demonstrates a 1-year survival with systemic palliative care, contributing to the evolving knowledge surrounding EACC.